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Biomedical subjects

J C Job

Publications and source records attributed to J C Job.

At least 109 records · Page 6Linked to original sources

Somatomedin, transferrin and amino-acids in serum following injection of human growth hormone in children with growth disease.

17 children with growth retardation (12 with idiopathic hypopituitary dwarfism, 2 with craniopharyngioma and 3 constitutionally short) were studied for three days following a single intramuscular injection of human growth hormone. Somatomedin activity was bioassayed using both sulphate incorporation into chick embryo cartilage and thymidine uptake by human lectin-activated lymphocytes. In hypopituitary patients it showed a significant response, maximal 24 hours after the injection, and significantly correlated for the two bioassays. The aminoacid content of the incubation medium used for thymidine bioassay appeared as an important factor: both glutamine and nonessential aminoacids are required to obtain significant stimulation by low serum concentrations, thus increasing the sensitivity of the assay but reducing the differences between normal and hypopituitary sera. Transferrin levels in serum were significantly lower in hypopituitary dwarfs. They did not rise in the three days following hGH. Aminoacid levels were lower in idiopathic GH deficient patients than in other groups, and did not show short term increase in the fasting samples collected after hGH administration.

Adolescent↗

Plasma growth-promoting activity measured as thymidine activity in constitutionally short children.

Growth-stimulating activity of plasma was measured, both as thymidine activity using a lymphocyte bioassay and as sulfation activity using a cartilage bioassay, in 72 constitutionally short children aged 1-6 years and 38 age-matched controls. Plasma transferrin was also measured. No significant differences were found between the two groups. However, short children aged 1-3 years did not have higher thymidine activity than children aged 4-10 years, whereas in normals the thymidine activity was significantly higher in the first years of life. Thymidine activity was lower in female than in male constitutionally short children.

Adolescent↗

Growth hormone dependency of the effect of human serum on thymidine uptake by lectin-activated human lymphocytes.

This study of the effect of serum dialysis on thymidine incorporation into lectin-activated blood lymphocytes cultured in vitro showed that (1) after serum dialysis 3H-thymidine incorporation is very low or absent; (2) the addition of L-glutamine to dialyzed sera leads to recovery of 18% of 3H-thymidine uptake, and (3) addition of L-glutamine reduces the difference in the stimulating effects of acromegalic and hypopituitary sera observed before dialysis in a medium free of L-glutamine. Since the plasma level of glutamine is increased in acromegalics and reduced in hypopituitary patients, it is suggested that this amino acid acts as a growth hormone dependent factor or cofactor in the regulation of cell growth.

Acromegaly↗

Hyporeninemic hypoaldosteronism in infancy: a familial disease.

Hyporeninemic hypoaldosteronism was found in two male siblings with urinary salt wasting and low plasma sodium levels. The eldest, aged 1 yr, had growth retardation, with hyponatremia and normal plasma potassium levels. The second, aged 2 months, had low plasma sodium and high plasma potassium levels. Both were severely and repeatedly hypoaldosteronemic. Primary adrenal deficiency was excluded by ACTH testing, which showed a good aldosterone rise and normal responses of other steroids. Both children had low PRA compared to that in age-matched normal subjects. The eldest sibling also had decreased total renin, low inactive to total renin ratio, and a subnormal level of angiotensinogen. The father had low plasma angiotensinogen levels. Congenital deficiency of renin activity and/or angiotensinogen production is suggested as the primary abnormality.

Adrenal Cortex Hormones↗

Studies in constitutionally tall adolescents. II. Effects of bromocriptine on growth hormone secretion and adult height prediction.

After previous demonstration of paradoxical GH responses to TRH and oral glucose loading tests in some constitutionally tall adolescents, we studied the effects of a small dose of bromocriptine (5 mg/day) on GH secretion and adult height prediction. In 10 patients, each of whom had a substantial plasma GH increase after TRH injection, bromocriptine reduced or suppressed this abnormal response. In 4 of these patients who also had paradoxical GH increases after glucose loading, bromocriptine suppressed this abnormality in 2. Predicted adult height was reduced in 10 of the 12 patients after 6-12 months of treatment. This reduction resulted either from a decrease in growth velocity, an increase in skeletal maturation rate, or both. Since no side effects were noticed we suggest that bromocriptine may be a valuable alternative to sex steroid treatment to limit final height in excessively tall adolescents.

Adolescent↗

[Direct assay of free thyroxine in children. Application to the monitoring of the treatment of hypothyroidism].

A direct measurement of free thyroxine (FT4) by a radio-immunologic method using an analogue of thyroxine as tracer allowed to establish the normal values in 341 children from birth to puberty. These values were used as reference for the determination of free T4 in the diagnosis and management of treatment for congenital hypothyroidism. In 44 treated hypothyroid children, there was a positive correlation between the serum concentration of free T4 and the amount of levothyroxine given daily (r = 0.46, p less than 0.01). There was also a negative correlation between free T4 and serum TSH (r = 0.59, p less than 0.001). Thus, the measurement of free thyroxine seems to be a more reliable, more accurate and more sensitive parameter than that of total thyroxine or TSH for controlling the treatment of congenital hypothyroidism.

Adolescent↗

[Thyroid pathology of newborn infants of mothers with Basedow's disease].

Of 15 neonates born to mothers presenting with Graves' disease, who were admitted for presumptive thyroid disorder, 6 presented with neonatal thyrotoxicosis, 4 with goiter and/or hypothyroidism, and 5 presented with euthyroidism. In cases with thyrotoxicosis, the mothers were not or insufficiently treated. Evolution was regressive in 5 instances, there was a craniostenosis and persisting hyperthyroidism in one case. In cases with goiter and/or hypothyroidism, on the other hand, the mothers were correctly treated, and neonatal manifestations were benign and transient. This emphasizes both the importance of supervising gravidic hyperthyroidism and the complexity of materno-foetal relationship in thyroid diseases.

Age Factors↗

Growth hormone response to thyrotropin-releasing hormone and oral glucose-loading tests in tall children and adolescents.

The effects of TRH and oral glucose loading on the release of GH were investigated in 10 children or adolescents with constitutional tall stature. Eight of these children had a family history of above average height. The mean (and SEM) baseline GH levels (3.12 +/- 2.0 ng/ml) were similar to those in control subjects. Somatomedin activity, measured by sulfate incorporation into chick embryo cartilage, was elevated. Oral glucose loading caused an early significant increase in plasma GH at 30 min in 2 of these subjects and a late rise at 180 min in 4 others. Intravenous injection of synthetic TRH (0.2 mg/m2) caused a marked increase in plasma GH (17 +/- 3.0 ng/ml) in 7 of the 10 patients; the peak of GH was observed within the first hour after the injection of TRH in 3 cases, while a later peak was observed in the second hour after injection in 4 others. Peak plasma PRL (47 +/- 3.8 ng/ml) and TSH (20 +/- 1.2 microU/ml) levels in response to TRH were normal. These results suggest a disorder of hypothalamo-pituitary regulation of GH secretion in certain children with apparently constitutional tall stature. Similar findings in a probably preacromegalic girl are reported.

Administration, Oral↗

Glucose metabolism in hyperinsulinemic infants: the effects of fasting and sodium DL-beta-hydroxybutyrate on glucose production and utilization rates.

Glucose metabolism was investigated in four infants aged 3-32 months with persistent hypoglycemia and hyperinsulinism of neonatal onset. Fasting hypoglycemia was found to be due both to an insulin-induced decrease in hepatic glucose output to 3.95 +/- 0.30 (SEM) mg/kg X min, a value about two thirds of normal, and to a glucose utilization rate of 4.25 +/- 0.32 mg/kg X min, which exceeded glucose production by about 8%. Simultaneously, and despite hypoglycemia, fasting plasma D-beta-hydroxybutyric acid concentrations were inappropriately low: 406 +/- 146 microM, presumably the result of elevated circulating insulin levels. The infusion of sodium DL-beta-hydroxybutyrate resulted in an increase of plasma glucose (48 +/- 7 vs. 32 +/- 7 mg/dl, P less than 0.01) and lactate (1704 +/- 217 vs. 964 +/- 149 microM, P less than 0.005), without detectable changes in insulin secretion estimated from circulating C-peptide values. Unexpectedly, the increase of plasma glucose was due to the restoration of glucose production up to 6.7 +/- 0.2 mg/kg X min. The individual increments of plasma lactate and glucose production rate were linearly correlated (P less than 0.01). These results together with the known inhibitory effect of ketone bodies on pyruvate dehydrogenation, suggest both increased production of lactate from peripheral recycling of glucose carbon and an increased conversion of this gluconeogenic precursor into glucose.

3-Hydroxybutyric Acid↗

Prolactin and thyrotrophin responses to thyroliberin (TRH) in patients with growth hormone deficiency: study in 167 patients.

Both thyrotrophin (TSH) and prolactin (Prl) were studied under thyroliberin (TRH) stimulation tests in 167 hypopituitary dwarfs out of GH or T4 treatment. TSH and/or Prl responses were either low, normal or exaggerated and/or protracted. Various abnormal patterns were observed in most of the patients with low T4 but also in many patients with normal T4. The TSH response should be considered together with the value of T4. A normal response of TSH with a low T4 reflects a relative TSH deficiency from pituitary or hypothalamic origin. There was no clear relationship between the cause or type of hypopituitarism and the pattern of the responses of either TSH or Prl. The abnormalities of TSH and Prl were not related to each other except in patients with a past history of breech delivery. Then both TSH and Prl have to be measured after TRH in order to obtain full information from the test about hypothalamo-pituitary function. The frequency of the exaggerated and/or delayed or protracted responses of TSH and Prl with normal or low T4 is probably mostly related to hypothalamo-pituitary dysfunction. Abnormal responses of TSH or Prl, seldom of both hormones, were observed in otherwise isolated growth hormone (GH) deficiency, leading to a modification of such a diagnosis after the TRH test. Actually, the TRH test may be useful to ascertain the diagnosis of GH deficiency when the GH responses to provocative tests are borderline.

Adolescent↗

[Precocious puberty in boys. Study of a series of 34 cases].

Thirty-four boys with true precocious puberty were observed: In 28, precocious puberty was related to intracranial causes (17 tumors, 11 other lesions), in 3, it was due to extrapituitary gonadotropin-secreting tumors and in 3 it appeared idiopathic. The clinical development of sex characteristics was slower than the advance of bone age and the increase in hormone levels (plasma testosterone and pituitary gonadotropin reserve as measured by the LH-RH test). Cyproterone treatment seemed more effective than medroxyprogesterone, with respect to both sexual development and evolution of the bone age/height age ratio or the predictable adult height.

Body Height↗

[Isolated hypothyroid dwarfism. Study of 18 cases].

Isolated hypothyroid dwarfism is defined by growth retardation without other clinical symptoms or signs of thyroid insufficiency. This study reports 18 cases: 6% of a series of cases of hypothyroidism in childhood. The main features are: mean bone age lower than height age; low plasma thyroxine in 16 patients, definitely low triiodothyronine in one only, high plasma TSH in all cases, with exaggerated response to TRH stimulation test; enlarged sella turcica in 11 patients. The thyroid gland was ectopic in 14 patients, the 4 others had a thyroid in normal place. Treatment with thyroid hormones sharply increased the growth velocity and the bone maturation. However its delay did not allow to obtain a complete catch-up of height.

Adolescent↗

[Treatment of congenital hypothyroidism by L-thyroxine in solution. Posology].

A solution of levothyroxine (5 micrograms/drop) has been used in congenital hypothyroidism detected after neonatal screening. Optimal doses were variable among individual patients. With 5-7 micrograms/kg during the first month of treatment, then 4 to 5 micrograms/kg, it was usually possible to obtaining normal plasma T4, which seems more important that to normalize TSH. Overdosage is to be avoided as well as insufficiency of treatment.

Administration, Oral↗

Results of early treatment of cryptorchidism with human chorionic gonadotropin.

One hundred and fifty-three children with common cryptorchidism, 109 unilateral and 44 bilateral, excluding those with associated malformations or abnormalities, were treated at age 6 to 59 months with human chorionic gonadotropin given as nine intramuscular injections on alternate days. Treatment before age 3 years resulted in complete failure in 81%. At 3 to 4 years of age treatment resulted in failure in 55%, but 19% of the patients showed complete testicular descent and 26% showed partial descent. The percent of failures was increased when the dose of human chorionic gonadotropin was lower than 1,000 IU/m2 injection and when the cryptorchid testis was very high. No correlation was found between endocrine data and the clinical results. The plasma testosterone concentration after the third injection of human chorionic gonadotropin was not significantly different in successfully and unsuccessfully treated patients. However, testosterone levels were significantly lower in patients treated at 36 to 59 months of age than in those treated at an earlier age, in contrast to the significantly better clinical results obtained in the older group. Thus human chorionic gonadotropin is not a valuable means of obtaining descent of undescended testes before age 3 years and is of limited usefulness at age 3 to 4 years.

Age Factors↗